Unusual Relapse of Chronic Lymphocytic Leukemia After Remission

  • Rizvi W
  • Truong Q
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Background: Chronic Lymphocytic Leukemia (CLL) is the most prevalent leukemia with over 20,000 estimated cases in 2017. Leukemic involvement of the nervous system causing neurological symptoms is reported in only about 1% of CLL patients. Unfortunately, there is no current standard therapy for treatment of CLL leptomeningeal disease. In this case, we discuss an unusual presentation of CLL leptomeningeal disease misdiagnosed as chronic rebound headache. Case Case Report: A 61-year- old female was diagnosed with Rai stage I CLL in 2002. When presented with peripheral blood lymphocytosis and subsequent flow cytometry revealed a mature B cell population consistent with CLL. The patient was monitored clinically as there were no indications for therapy. In 2006, the patient developed B symptoms along with hemolytic anemia refractory to steroids and was initiated on chemotherapy with fludarabine, cyclophosphamide, and rituximab (FCR), showing complete response after six cycles. The patient was in her usual state of health until 2016, when complained of chronic headaches. Tylenol and ibuprofen were administered regularly and the patient was diagnosed with rebound headaches by neurology. These symptoms progressed and the patient developed encephalopathy requiring inpatient admission. Magnetic Resonance Imaging (MRI) revealed abnormal enhancement in the cerebellar peduncles and dentate nuclei symmetrically and lumbar puncture performed revealed evidence of CLL consistent with leptomeningeal disease. Therapy was started with oral ibrutinib at 560 mg daily for better central nervous system penetration. After three months of therapy, the patient had complete resolution of symptoms and MRI abnormalities. Conclusion: Leptomeningeal disease is a rare complication of CLL that clinicians should be aware of and ibrutinib can be an effective, tolerable therapy for this debilitating disease.




Rizvi, W., & Truong, Q. (2018). Unusual Relapse of Chronic Lymphocytic Leukemia After Remission. Cureus. https://doi.org/10.7759/cureus.2176

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